[胰腺腺泡细胞癌的脂肪酶分泌过多综合征和骨病变。报告一例].
[Lipase hypersecretion syndrome and bone lesions in acinar cell cancer of the pancreas. Report of one case].
发表日期:2022 Jul
作者:
Eduardo Botello-Correa, Tomás Zamora-Helo, Pedro Osorio-Valdivia, María Emilia Riquelme-Stagnaro, María José Figueroa-Sepúlveda
来源:
Bone & Joint Journal
摘要:
脂肪酶分泌过多综合征 (LHS) 是一种罕见的副肿瘤综合征,10% 至 15% 的患者与胰腺腺泡细胞癌 (ACCP) 相关。临床上,LHS表现为皮下纤维囊性结节的出现,与上覆皮肤的营养性变化有关,例如难以处理的溃疡或瘘管,主要影响下肢。此外,关节附近和骨内脂肪组织中的脂肪分解可引起双侧关节痛,尤其是膝盖和脚踝。我们报告了一名 57 岁男性,有胰岛素抵抗和过敏性鼻炎病史,于 2019 年 6 月就诊,下肢(主要是双脚踝)出现多个皮下结节,与该区域的关节痛相关。此外,腹部 CT 扫描显示腹部有一明显肿块,尺寸约为 17 厘米,与胰体和胰尾接触,病理学上符合 ACCP。卡培他滨治疗一开始就取得了良好的进展。该患者目前存在左侧踝后小瘘,根据分析、研究和文献回顾,该瘘属于 LHS 背景下的病变。
Lipase hypersecretion syndrome (LHS) is a rare paraneoplastic syndrome, associated with acinar cell carcinoma of the pancreas (ACCP) in 10% to 15% of patients. Clinically, LHS manifests itself with the appearance of subcutaneous fibrocystic nodules, associated with trophic changes in the overlying skin, such as ulcers or fistulas that are difficult to manage, mainly affecting the lower extremities. Additionally, lipolysis near the joints and in the intraosseous adipose tissue can cause bilateral arthralgias, especially of the knees and ankles. We report a 57-year-old man, with a history of insulin resistance and allergic rhinitis, who presented in June 2019 with multiple subcutaneous nodules in the lower extremities, predominantly in both ankles, associated with arthralgia in that region. Additionally, a CT scan of the abdomen revealed a significant abdominal mass, measuring approximately 17 cm and in contact with the body and tail of the pancreas, pathologically compatible with an ACCP. Treatment with capecitabine was started with a favorable progression. The patient currently presents a small left lateral retro malleolar fistula, which, given the analyzes, studies and reviewed literature is concluded to be a lesion in the context of LHS.